About 50% of ARVC patients carry a mutation in genes encoding one of the five essential cardiac desmosomal proteins: the desmosomal cadherins desmoglein (DSG)-2 and desmocollin-2 and the armadillo repeat-containing proteins plakoglobin and plakophilin (PKP)-2, as well as the plaque protein desmoplakin ([6,8,17], graphical abstract). This evidence concerns the gene DSG2 and Arrhythmogenic right ventricular dysplasia.